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angiofollicular lymph node hyperplasia; Castleman lymphadenopathy
Currently considered to be a systemic disease; may be multicentric.
Etiology: unknown. Some cases associated with human herpes virus type 8.
Microscopic Pathology: enlarged lymph nodes, two pattern types
1) hyaline vascular type: solitary masses of lymphoid tissue containing concentric perivascular aggregates of lymphocytes
2) plasma cell type: sheets of interfollicular plasma cells
Clinical manifestations:
1) lymphadenopathy
a) abdomen or mediastinum (younger patients)
b) multicentric (older patients)
c) hepatosplenomegaly
2) case presented with hematuria after hiking [5]
3) retroperitoneal mass (case report) [5]
Laboratory:
1) complete blood count (CBC) may show anemia
2) lymph node biopsy
Radiology:
- chest X-ray
- hilar or mediastinal adenopathy
- abdominal ultrasound -> abdominal CT
- magnetic resonance imaging (MRI) of the abdomen & pelvis annually in follow-up [5]
Differential diagnosis:
1) Castleman like changes may be seen in
a) autoimmune disease
b) primary immune deficiencies
c) Kaposi sarcoma
d) late phase of HIV-associated adenopathy
2) large accumulations of plasma cells is nonspecific and may be seen in
a) rheumatoid arthritis
b) lues
c) angioimmunoblastic lymphadenopathy
d) POEMS syndrome
3) lymphoma
Complications:
-> may progress to lymphoma or plasmacytoma
Management:
- surgical resection as indicated [5]
- radiation therapy [5]
General
lymphoid tissue disease; lymphoid disorder
References
- Stedman's Medical Dictionary 26th ed, Williams &
Wilkins, Baltimore, 1995
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY,
1994, pg 324
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
- Ioachim's Lymph Node Pathology. Ioachin & Ratech eds.,
Lippincott, Williams & Wilkins, 2002. Chap 43.
- Blute ML et al
Case 5-2017 - A 19-Year-Old Man with Hematuria and a
Retroperitoneal Mass.
N Engl J Med 2017; 376:684-692. February 16, 2017
PMID: 28199810
http://www.nejm.org/doi/full/10.1056/NEJMcpc1610100